Safety & feasibility of responsive neurostimulation in children with refractory epilepsy: A single-center experience.

OBJECTIVES: Responsive neurostimulation (RNS) is a relatively recent addition to the epilepsy surgery armory, gaining FDA approval in 2013 for use in adults with intractable focal epilepsy. Data for the use of RNS system in patients less than 18 years of age is limited. We aim to determine the safety and feasibility of RNS in children with refractory epilepsy. METHODS: A retrospective chart review was conducted for all patients who underwent RNS implantation at an urban tertiary children's hospital. Demographics of the patients were obtained, including age at the time of implant, MRI findings, seizure onset zone identification, and RNS targets. RESULTS: Out of a fourteen patient cohort, one patient had a post-operative complication of infection at surgical site requiring explantation. Thirteen out of 14 patients had immediate post-operative head imaging that was negative for hemorrhage, infarction, or skull fracture; one patient did not undergo head imaging. No patients reported a worsening clinical seizure frequency at a 6-month follow up visit. In the subset of patients who were implanted with RNS and did not undergo concurrent resections, there was a statistically significant reduction in the average number of long episodes at the most recent visit when compared to the 1-month post-operative visit (p = 0.0268). CONCLUSIONS: RNS is a feasible and safe option for children as young as six years with refractory epilepsy when appropriate seizure focus identification has been performed with stereo CT and stereo EEG evaluations, and can be used in conjunction with other surgical epilepsy treatment modalities. Two canister RNS placement is achievable for patients with a broad epileptogenic network or multifocal seizure onset zones.

Patients Diagnosed with Multisystem Inflammatory Syndrome in Children Have Persistent Neurologic, Sleep, and Psychiatric Symptoms After Hospitalization.

Background: Acute neurological complications from COVID-19 have been reported in both pediatric and adult populations. Chronic symptoms after recovery have been reported in adults and can include neuropsychiatric and sleep symptoms. Persistent symptoms in children with the multisystem inflammatory syndrome in children (MIS-C) have not been studied. Methods: We conducted a single-center retrospective chart review and cross-sectional survey of patients diagnosed with MIS-C. Patients and parents were surveyed on symptoms before the COVID-19 pandemic, upon admission, and 23 weeks (interquartile range 20-26 weeks) after discharge. Age and gender-matched patients requiring intensive care unit (ICU) care for status asthmaticus were surveyed as a control group. Results: In this cohort of 47 patients, 77% reported neurological, 60% psychiatric, and 77% sleep symptoms during hospitalization. Prior to hospitalization, 15% reported neurological, 0% psychiatric, and 7% sleep symptoms. Eighteen (50%) of the 36 patients who had neurological symptoms during hospitalization continued to have symptoms on follow-up (odds ratio [OR] = ∞, p = .003]). Similarly, 16 (57%) of 28 patients with psychiatric symptoms reported persistence at follow-up (OR = 5.00; p = .02). Fifteen (42%) of the 18 patients reporting sleep disturbance during hospitalization had persistence on follow-up (OR = 1.9; p = .49). The aggregate of neurological, psychiatric, and sleep symptoms during admission and at follow-up was significantly higher for MIS-C patients requiring ICU care when compared to the control group (p = .01). Conclusions: In this cohort of patients with MIS-C, a majority of patients reported new-onset neuropsychiatric and sleep symptoms. Almost half of these patients had persistent symptoms on a follow-up survey.

Sudden unexpected death in children: myth or reality?

PURPOSE OF REVIEW: Despite many years of study, sudden unexplained death remains a tenuous diagnosis of exclusion. Here, we discuss the current science behind the uncertainties of sudden death, as well as the questions that still remain. RECENT FINDINGS: Failure in any part of the complex interplay between peripheral sensors and central cardiorespiratory regulation can result in sudden death. Diagnostic testing with electrocardiograms, electroencephalogram, sleep studies, or even genetic studies have increased our ability to identify patients at the highest risk. SUMMARY: Advances in the understanding of sudden unexplained death in children may show common pathways leading to sudden death from multiple different diseases. Although rare, the devastating implication prioritizes the importance in educating patients about how to live with the risk of sudden death.

Autism: considerations for transitions of care into adulthood.

PURPOSE OF REVIEW: The steady rise in number of youth diagnosed with autism spectrum disorder (ASD) has led to the need to examine transition of care considerations specific to ASD. Improved understanding and guidance addressing these needs will allow pediatric and adult providers to work together to optimize social, medical, and occupational outcomes for these patients. RECENT FINDINGS: Health-care transition is a delicate time when children with ASD outgrow the services of pediatric programs and enter a fragmented healthcare system that is unfamiliar, insufficiently knowledgeable, and underfunded for their needs. SUMMARY: Increasing autism prevalence and an aging population with autism lend urgency to improve outcomes in children transitioning to adult-care. Research reveals poor consequences in social support, education, vocational training and employment, housing, and healthcare. Specific considerations to address these issues and ensure successful transition from pediatric to adult care are needed.

A Rare Syndrome of GRID2 Deletion in 2 Siblings.

The Glutamate receptor, ionotropic, delta 2 gene codes for an ionotropic glutamate delta-2 receptor, which is selectively expressed in cerebellar Purkinje cells, and facilitates cerebellar synapse organization and transmission. The phenotype associated with the deletion of Glutamate receptor, ionotropic, delta 2 gene in humans was initially defined in 2013. In this case report, the authors describe 2 brothers who presented with developmental delay, tonic upward gaze, nystagmus, oculomotor apraxia, hypotonia, hyperreflexia, and ataxia. They were found to have a homozygous intragenic deletion within the Glutamate receptor, ionotropic, delta 2 gene at exon 2. Our patients serve as an addition to the literature of previously reported children with this rare clinical syndrome associated with Glutamate receptor, ionotropic, delta 2 deletion.